2q5h
From Proteopedia
(New page: 200px<br /> <applet load="2q5h" size="450" color="white" frame="true" align="right" spinBox="true" caption="2q5h, resolution 3.0Å" /> '''Crystal structure of...) |
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| - | [[Image:2q5h. | + | [[Image:2q5h.jpg|left|200px]]<br /><applet load="2q5h" size="350" color="white" frame="true" align="right" spinBox="true" |
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caption="2q5h, resolution 3.0Å" /> | caption="2q5h, resolution 3.0Å" /> | ||
'''Crystal structure of apo-wildtype Glycyl-tRNA synthetase'''<br /> | '''Crystal structure of apo-wildtype Glycyl-tRNA synthetase'''<br /> | ||
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==Overview== | ==Overview== | ||
Dominant mutations in the ubiquitous enzyme glycyl-tRNA synthetase, (GlyRS), including S581L, lead to motor nerve degeneration. We have, determined crystal structures of wildtype and S581L-mutant human GlyRS., The S581L mutation is approximately 50A from the active site, and yet, gives reduced aminoacylation activity. The overall structures of wildtype, and S581L-GlyRS, including the active site, are very similar. However, residues 567-575 of the anticodon-binding domain shift position and in, turn could indirectly affect glycine binding via the tRNA or alternatively, inhibit conformational changes. Reduced enzyme activity may underlie, neuronal degeneration, although a dominant-negative effect is more likely, in this autosomal dominant disorder. | Dominant mutations in the ubiquitous enzyme glycyl-tRNA synthetase, (GlyRS), including S581L, lead to motor nerve degeneration. We have, determined crystal structures of wildtype and S581L-mutant human GlyRS., The S581L mutation is approximately 50A from the active site, and yet, gives reduced aminoacylation activity. The overall structures of wildtype, and S581L-GlyRS, including the active site, are very similar. However, residues 567-575 of the anticodon-binding domain shift position and in, turn could indirectly affect glycine binding via the tRNA or alternatively, inhibit conformational changes. Reduced enzyme activity may underlie, neuronal degeneration, although a dominant-negative effect is more likely, in this autosomal dominant disorder. | ||
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| - | ==Disease== | ||
| - | Known diseases associated with this structure: Charcot-Marie-Tooth disease, type 2D OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=600287 600287]], Neuropathy, distal hereditary motor, type V OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=600287 600287]] | ||
==About this Structure== | ==About this Structure== | ||
| - | 2Q5H is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Active as [http://en.wikipedia.org/wiki/Glycine--tRNA_ligase Glycine--tRNA ligase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=6.1.1.14 6.1.1.14] Full crystallographic information is available from [http:// | + | 2Q5H is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Active as [http://en.wikipedia.org/wiki/Glycine--tRNA_ligase Glycine--tRNA ligase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=6.1.1.14 6.1.1.14] Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2Q5H OCA]. |
==Reference== | ==Reference== | ||
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[[Category: structural genomics]] | [[Category: structural genomics]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Jan 23 14:23:26 2008'' |
Revision as of 12:23, 23 January 2008
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Crystal structure of apo-wildtype Glycyl-tRNA synthetase
Overview
Dominant mutations in the ubiquitous enzyme glycyl-tRNA synthetase, (GlyRS), including S581L, lead to motor nerve degeneration. We have, determined crystal structures of wildtype and S581L-mutant human GlyRS., The S581L mutation is approximately 50A from the active site, and yet, gives reduced aminoacylation activity. The overall structures of wildtype, and S581L-GlyRS, including the active site, are very similar. However, residues 567-575 of the anticodon-binding domain shift position and in, turn could indirectly affect glycine binding via the tRNA or alternatively, inhibit conformational changes. Reduced enzyme activity may underlie, neuronal degeneration, although a dominant-negative effect is more likely, in this autosomal dominant disorder.
About this Structure
2Q5H is a Single protein structure of sequence from Homo sapiens. Active as Glycine--tRNA ligase, with EC number 6.1.1.14 Full crystallographic information is available from OCA.
Reference
Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase, an enzyme underlying distal spinal muscular atrophy., Cader MZ, Ren J, James PA, Bird LE, Talbot K, Stammers DK, FEBS Lett. 2007 Jun 26;581(16):2959-2964. Epub 2007 May 29. PMID:17544401
Page seeded by OCA on Wed Jan 23 14:23:26 2008
Categories: Glycine--tRNA ligase | Homo sapiens | Single protein | Bird, L.E. | Cader, M.Z. | James, P.A. | OPPF, Oxford.Protein.Production.Facility. | Ren, J. | Stammers, D.K. | Talbot, K. | Aminoacyl-trna synthetase | Atp-binding | Glycyl-trna synthetase | Oppf | Oxford protein production facility | Structural genomics
